ABSTRACT
BACKGROUND: Irritability, depression and apathy are frequently reported neuropsychiatric symptoms of Huntington's disease (HD). OBJECTIVE: This study investigated the course of irritability, depression and apathy in HD during a 2-year follow-up period. METHODS: In 121 HD mutation carriers the presence of irritability, depression and apathy was measured with the Problem Behaviours Assessment (PBA). Multivariate linear regression analysis was performed to assess their relationships with the change of the motor score of the Unified Huntington's Disease Rating Scale (UHDRS-m) in premotor symptomatic (n = 46) and motor symptomatic mutation carriers (n = 75). RESULTS: The median depression score of all participants decreased (p = 0.002), whereas irritability and apathy scores did not change significantly. In the total group of mutation carriers, a borderline significant association was found between an increase in motor symptoms and an increase in irritability (p = 0.05), and a trend was found for the association between an increase in motor symptoms and a decrease in depression (p = 0.06). Only in the at baseline premotor symptomatic mutation carriers was an increase in motor symptoms significantly related to an increase in irritability (p = 0.02). CONCLUSION: An increase in motor symptoms in the at baseline premotor symptomatic mutation carriers is related to an increase in irritability, which may be an early and sensitive marker for disease progression.
Subject(s)
Apathy , Depression , Huntington Disease/psychology , Irritable Mood , Adult , Depression/genetics , Disease Progression , Female , Follow-Up Studies , Humans , Huntington Disease/genetics , Male , Middle Aged , Psychiatric Status Rating Scales , Severity of Illness IndexABSTRACT
Irritability is a frequent neuropsychiatric symptom in patients with Huntington's disease (HD). The Irritability Scale (IS) and the irritability factor of the Problem Behaviours Assessment (PBA) was used to assess irritability among 130 HD mutation carriers and 43 verified non-carriers. The IS was tested using receiver operating characteristic analysis against different cut-offs of the PBA irritability factor. A robust IS cut-off score of ≥14 points was found indicating that 45 (35%) of the 130 mutation carriers were irritable vs. 4 (9%) of the 43 non-carriers (P=0.001). The level of agreement between self-report and informant-report IS was of moderate strength (intraclass correlation=0.61). Using univariate and multivariate regression analyses, independent correlates of irritability were being married/living together (P=0.02), CAG repeat length (P=0.01), and use of benzodiazepines (P=0.008). Using the same model with the informant's irritability score, use of benzodiazepines was the only significant independent correlate of irritability (P=0.005). Irritability is a prominent symptom of HD and can be reliably assessed with the IS using a cut-off score ≥14 points. Although it is unclear whether benzodiazepine use causes irritability, or irritability leads to the prescription of benzodiazepines, clinical evaluation with respect to the use of benzodiazepines in HD warrants attention.
Subject(s)
Huntington Disease/psychology , Irritable Mood , Adult , Aged , Female , Humans , Male , Middle Aged , Psychiatric Status Rating Scales , Psychometrics , Self ReportABSTRACT
This study examined the incidence and course of apathy in subjects with Huntington's disease (HD). Our results showed that at follow-up 14% of the subjects free of apathy at baseline had developed apathy. In these subjects, a lower baseline Mini-Mental State Exam score predicted incidence of apathy. Of the 34 subjects with apathy at baseline, 14 subjects were no longer apathetic at follow-up. Twenty subjects had persistent apathy, with a low baseline Symbol Digits Modalities Test as the only predictor. These results showed that apathy in HD is most closely linked to global and executive cognitive performance.
Subject(s)
Apathy , Huntington Disease , Adult , Female , Humans , Huntington Disease/diagnosis , Huntington Disease/epidemiology , Huntington Disease/physiopathology , Incidence , Logistic Models , Longitudinal Studies , Male , Middle Aged , Neuropsychological Tests , Predictive Value of Tests , Retrospective Studies , Severity of Illness IndexABSTRACT
Besides chorea, hypokinesia is an important motor disturbance in Huntington's disease (HD) but its clinical, neuropsychiatric, and cognitive functioning correlates are largely unknown. This cross-sectional study investigates correlates of hypokinesia in HD and its effect on global functioning. Among 150 HD gene carriers, 96 patients were clinically motor manifest. Hypokinesia was assessed using the motor section of the Unified Huntington's Disease Rating Scale and global functioning was measured using the Total Functioning Capacity (TFC) scale. Neuropsychiatric measures included the Apathy Scale and the Composite International Diagnostic Interview for diagnosis of depression. The Mini Mental State Examination (MMSE) and a composite executive cognitive measure were used to assess global and executive cognitive functioning, respectively. Compared with 45 patients with no or mild hypokinesia, 51 patients with moderate to severe hypokinesia showed a significant difference in most clinical and neuropsychiatric variables and had worse cognitive functioning scores. However, using forward logistic regression analysis, poor executive cognitive functioning was the only independent correlate of hypokinesia (OR 7.33; 95% CI: 2.82-19.0; P < 0.001). Hypokinesia score was inversely associated with the TFC score (P < 0.001), also after adjusting for chorea, use of antipsychotics, apathy, and global and executive cognitive functioning. In conclusion, the presence of moderate to severe hypokinesia in HD patients co-occurs with executive cognitive dysfunction and adversely affects global functioning.
